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Cellular Angiofibroma

Definition

  • Circumscribed inguinal / perineal mass composed of bland spindled cells will prominent medium sized round vascular component

Alternate / historical names

  • Angiomyofibroblastoma-like tumor of male genital tract

Diagnostic Criteria

  • Well circumscribed subcutaneous nodule
    • May have thin fibrous capsule
    • Predominantly dermal in 10% of cases
    • 2/51 cases reported with infiltration
  • Uniform distribution of bland spindle cells
    • Moderately cellular
    • Low mitotic rate, usually <1/10 hpf
    • Occasional findings
      • Mitotic rate up to 10/10 hpf in 10% of cases
      • Intranuclear inclusions and nuclear grooves
      • Vague nuclear palisading
  • Prominent hyalinized round vessels
    • Small to medium size
    • Generally evenly distributed
    • Staghorn vessels focal about 10% of cases
  • Fat admixed in 25% of cases
    • May make up 50% of lesion
  • Fine collagen bundles
    • Edematous to fibrous stroma
  • One series of 13 cases reported with atypia or sarcomatous transformation (Chen)
    • Typical areas of cellular angiofibroma present
    • 12/13 female
    • 4 with atypia
      • Large hyperchromatic cells
      • 3 cases with scattered foci, 1 with a discrete nodule
    • 9 with sarcomatous transformation
      • 2 with pleomorphic liposarcoma
      • 3 resembling atypical liposarcoma
        • MDM2 and CDK4 negative
      • 4 with abrupt transition to pleomorphic spindle cells
    • p16 multifocal or diffuse in atypical cells or sarcomatoid areas
      • Scattered weak to negative in ordinary cellular fibroma areas
    • Same behavior so far as usual cellular fibroma
      • No recurrences or metastases

Richard L Kempson MD
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342

Original posting/updates: 5/27/07, 6/18/10

 

Supplemental studies

Immunohistology

Vimentin positive
CD34 29/48
Smooth muscle actin 10/48
Desmin 4/48
Estrogen receptor 35%
Progesterone receptor 55%
CD10 2/7
S100 negative

(EMA one case reported positive)

p16 multifocal or diffuse in cases with atypical cells or sarcomatoid areas

  • Scattered weak to negative in ordinary cellular fibroma areas

Differential Diagnosis

The first four entities are predominantly perineal in location and may be confused with cellular angiofibroma

Angiomyofibroblastoma Cellular Angiofibroma
Alternating hypercellular and hypocellular areas Uniformly moderately cellular
Numerous small vessels Hyalinized round vessels
Clusters of rounded, epithelioid cells, often around vessels Bland spindle cells
Desmin positive most cases Desmin about 10% of cases
Numerous small vessels Large thick walled vessels
Actin frequently positive Actin infrequently positive
Both may have fat in lesion, both may be CD34+.

Aggressive Angiomyxoma Cellular Angiofibroma
Usually large, deep lesion Subcutaneous, mean size 6 cm
Hypocellular Moderately cellular
Infiltrative margin Circumscribed
Usually desmin positive Desmin 10% positive
May infiltrate fat May have fat integral to lesion
Both have prominent medium sized vessels, both may be CD34+.

Superficial Myofibroblastoma Cellular Angiofibroma
Desmin positive Desmin 10% positive
Inconspicuous thin walled vessels Prominent thick walled vessels
No fat Frequently contains fat
ER and PR >90% ER and PR 35-55%
Both are superficial and may be CD34 postive. Cellular angofibroma may occur in males.

Mammary-type Myofibroblastoma Cellular Angiofibroma
Bundles of hyalinized collagen Wispy collagen
Vessels inconspicuous Vessels prominent
Desmin and CD34 generally positive Desmin and CD34 variable
Both may contain adipose tissue

Cellular fibroma is virtually restricted to perineal, genital, inguinal regions, while the entities considered below may occur in a wide variety of sites.

Solitary Fibrous Tumor Cellular Angiofibroma
Alternating cellular and hypocellular areas Generally uniformly cellular
Staghorn vessels prominent Hyalinized round vessels prominent
Hyalinized collagen bundles frequent Wispy collagen frequent
CD34+ nearly all cases CD34+ 60% of cases

Spindle Cell Lipoma Cellular Angiofibroma
CD34+ nearly all cases CD34+ 60% of cases
Essentially restricted to neck, upper back Nearly all inguinal or perineal
Ropy collagen Wispy collagen
Inconspicuous vessels Prominent vessels

Schwannoma Cellular Angiofibroma
S100 positive S100 negative

Superficial Angiomyxoma Cellular Angiofibroma
Long thin walled vessels Hyalinized round vessels
Stromal neutrophils Usually stromal lymphocytes

Fibroepithelial Stromal Polyp Cellular Angiofibroma
Exophytic Sessile
May have scattered atypical stromal cells Uniform cells in most cases

Leiomyoma Cellular Angiofibroma
Desmin, actin uniformly positive Desmin, actin variable
Spindled cytoplasm Scant cytoplasm

Clinical

  • Nearly all inguinal / perineal
    • In females, most vulvovaginal
    • In males, most inguinal or scrotal
    • Rare lesions in retroperitoneum, trunk, lumbar region
  • Even male : female incidence
  • Age 20-78 years
    • Female mean 46
    • Male mean 61
  • Size 0.6-25 cm, mean 5.7 cm
  • Subcutaneous 95% of cases
    • Predominantly dermal in 5%
  • Recurrences very rare
    • Metastases not reported
    • Same behavior reported for cases with atypical cells or sarcomatous transformation

Grading / Staging / Report

Grading and staging are not applicable

The surgical pathology report should contain or address the following:

  • Location
  • Type of resection or biopsy
  • Histologic diagnosis
  • Managerial category Ia (Local excision is almost always curative; metastasis never occurs)
  • Size
  • Depth (dermis, subcutis, below fascia, body cavity)
  • Margins
    • Involved
    • Not involved
  • Results of supplementary studies if performed
  • Relationship to other specimens from the same patient

Lists

Vulvovaginal stromal and spindled tumors

Lesions that may demonstrate a prominent hemangiopericytoma-like vascular pattern

Bibliography

  • Kempson RL, Fletcher CDM, Evans HL, Henrickson MR, Sibley RS. Tumors of the Soft Tissues, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 30, 2001
  • Fletcher CDM, Unni KK, Mertens F. Pathology and Genetics of Tumours of Soft Tissue and Bone, World Health Organization Classification of Tumours 2002
  • Weiss SW, Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors, 4th edition, 2001
  • Iwasa Y, Fletcher CD. Cellular angiofibroma: clinicopathologic and immunohistochemical analysis of 51 cases. Am J Surg Pathol. 2004 Nov;28(11):1426-35.
  • McCluggage WG, Ganesan R, Hirschowitz L, Rollason TP. Cellular angiofibroma and related fibromatous lesions of the vulva: report of a series of cases with a morphological spectrum wider than previously described. Histopathology. 2004 Oct;45(4):360-8.
  • Nucci MR, Granter SR, Fletcher CD. Cellular angiofibroma: a benign neoplasm distinct from angiomyofibroblastoma and spindle cell lipoma. Am J Surg Pathol. 1997 Jun;21(6):636-44.
  • Laskin WB, Fetsch JF, Mostofi FK. Angiomyofibroblastomalike tumor of the male genital tract: analysis of 11 cases with comparison to female angiomyofibroblastoma and spindle cell lipoma. Am J Surg Pathol. 1998 Jan;22(1):6-16.
  • Chen E, Fletcher CD. Cellular angiofibroma with atypia or sarcomatous transformation: clinicopathologic analysis of 13 cases. Am J Surg Pathol. 2010 May;34(5):707-14.
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